Incessant slow bundle branch reentrant ventricular tachycardia in a young patient with left ventricular noncompaction | Revista Portuguesa de Cardiologia (English edition)

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Both the left anterior descending artery and left circumflex artery are visualized and present no angiographic lesions. (A): Right anterior oblique with caudal angulation; (B): left lateral." ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Alexandra Sousa, Ricardo Lopes, João Carlos Silva, M. Júlia Maciel" "autores" => array:4 [ 0 => array:2 [ "nombre" => "Alexandra" "apellidos" => "Sousa" ] 1 => array:2 [ "nombre" => "Ricardo" "apellidos" => "Lopes" ] 2 => array:2 [ "nombre" => "João Carlos" "apellidos" => "Silva" ] 3 => array:2 [ "nombre" => "M. 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N. Gaia Hospital Centre, V. N. Gaia, Portugal" "etiqueta" => "c" "identificador" => "aff0015" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "pt" => array:1 [ "titulo" => "Taquicardia ventricular por reentrada de ramo lenta e incessante em adolescente com ventrículo esquerdo não-compactado" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0015" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 1481 "Ancho" => 2500 "Tamanyo" => 476579 ] ] "descripcion" => array:1 [ "en" => "Baseline ECG: sinus rhythm, intraventricular conduction defects and significant PR prolongation." ] ] ] "textoCompleto" => "IntroductionBundle branch reentrant ventricular tachycardia (BBR-VT), an uncommon form of macroreentrant tachycardia, generally occurs in the context of dilated cardiomyopathy, previous valve surgery or other cardiac conditions with underlying His-Purkinje system (HPS) disease. This case is a very unusual presentation of BBR-VT in a young patient with isolated left ventricular noncompaction (LVNC). Although our patient presented with HPS disease allowing initiation of this arrhythmia, it is rare for BBR-VT to be the first manifestation of isolated LVNC. Furthermore, BBR-VT is a highly malignant arrhythmia, yet our patient was almost asymptomatic due to the surprisingly long cycle length of the ventricular tachycardia (VT) and despite its unusual incessancy.Case reportA 15-year-old girl was referred to our arrhythmology department for mild palpitations and documented incessant VT (hemodynamically stable VT lasting hours). She was otherwise healthy, with no relevant medical history and no family history of significant cardiomyopathy or sudden cardiac death (SCD). Her palpitations were not related to effort and were persistent but otherwise extremely well tolerated. She denied precordial pain, dizziness, or presyncope/syncope. A previous electrocardiogram (ECG) had revealed wide-QRS tachycardia (WCT) at 115 beats per minute (bpm).InvestigationsThe patient was examined shortly after being referred, and denied any symptoms, including palpitations. The physical exam was unremarkable. The first ECG showed a VT with left bundle branch block (LBBB) pattern and superior axis at 118 bpm. A transthoracic echocardiogram revealed a normal-sized LV and preserved overall systolic function, hypertrabeculation of the LV posterior and lateral walls and intertrabecular recesses communicating with the LV cavity as demonstrated by color Doppler flow, suggestive of LVNC, which was confirmed by cardiac magnetic resonance imaging (<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>). Subsequent ECGs alternated between sinus rhythm with intraventricular conduction abnormalities and first-degree atrioventricular (AV) block and slow VT with LBBB pattern and superior axis (<a class="elsevierStyleCrossRef" href="#fig0010">Figure 2</a>).<elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia>An exercise stress test was stopped at 3:58 because of the sudden induction of a well tolerated yet sustained slow wide QRS tachycardia with right bundle branch (RBB) and left posterior fascicular block patterns. During most of the recovery time, an incomplete RBB block pattern with right axis deviation was seen, as at the beginning of the test.An electrophysiological study (EPS) was performed. The baseline ECG revealed sinus rhythm, intraventricular conduction defects (QRS 122 ms) and significant PR prolongation (296 ms) (<a class="elsevierStyleCrossRef" href="#fig0015">Figure 3</a>). A standard protocol using 6-F diagnostic electrophysiology catheters was followed. Two quadripolar catheters were placed in the high right atrium, His bundle and right ventricle as required, and a decapolar catheter in the coronary sinus. The programmed ventricular stimulation protocol included three drive-cycle lengths (CL) and two ventricular extrastimuli while pacing from the right ventricular apex. A 125 bpm-rate monomorphic sustained VT was reproducibly inducible with a single extrastimulus 360 ms after an eight-beat drive-cycle length of 600 ms. It had a LBBB pattern, superior axis and a clear right bundle deflection preceding each ventricular complex, suggesting the RBB was part of the circuit (<a class="elsevierStyleCrossRef" href="#fig0020">Figure 4</a>). A short postpacing interval was obtained from the right ventricular apex. Left posterior fascicular block was intermittently seen, associated with an increase in the VT CL, further suggesting a diagnosis of BBR-VT. The VT was exceptionally well tolerated and always terminated by anti-tachycardia pacing (ATP) with 380–420 ms CL. A second VT with a slightly different morphology in the limb leads was also inducible. Short periods of intermittent RBB block were documented during sinus rhythm. Further data included AH interval 157 ms; HV interval 100 ms; and Wenckebach period 540 ms (<a class="elsevierStyleCrossRef" href="#fig0025">Figure 5</a>).<elsevierMultimedia ident="fig0015"></elsevierMultimedia><elsevierMultimedia ident="fig0020"></elsevierMultimedia><elsevierMultimedia ident="fig0025"></elsevierMultimedia>TreatmentAs there was a considerable risk of need for chronic long-term ventricular pacing following RBB ablation and considering that this VT was particularly slow and practically asymptomatic, ablation of the RBB was not attempted and the patient was referred for implantation of a dual-chamber cardioverter-defibrillator (ICD). The rationale for this lay in the spontaneous occurrence of sustained VT, albeit slow, and the easy inducibility of sustained BBR-VT, a highly malignant ventricular tachyarrhythmia that had an unusual presentation in this patient but could nevertheless recur at higher rates. The ICD would protect the patient from potential future episodes of fast VT or ventricular fibrillation and would have additional advantages: (1) ventricular pacing in the event of complete AV block (the patient had significant bilateral bundle branch conduction abnormalities with marked HV prolongation); and (2) quantification of the number and duration of VT episodes in the upcoming months or years. If persistently high heart rates caused by long episodes of VT are observed, especially if associated with deterioration in LV systolic function (tachycardia-induced cardiomyopathy), RBB ablation will be considered. In this case, we will then opt for resynchronization.Outcome and follow-upDuring EPS and ICD implantation, ATP successfully terminated all episodes of VT. The defibrillator was accordingly programmed to deliver ATP for VT CL of 400–500 ms. Termination of episodes of slow VT could help prevent tachycardia-induced cardiomyopathy. A few hours after implantation, the patient received six shocks in a 2-hour period after failed ATP for relatively slow VT (rate 125 bpm), causing considerable distress to the patient, and ATP was turned off. No further ICD shocks were reported in the following six months and she remains asymptomatic. Several episodes of sustained slow VT have been detected by the ICD.DiscussionLVNC is a rare form of a primary genetic cardiomyopathy considered to be the result of abnormal intrauterine arrest of the myocardial compaction process.<a class="elsevierStyleCrossRef" href="#bib0005">1</a> Heart failure, arrhythmias (including SCD) and embolic events are its classical triad of complications. Ventricular tachyarrhythmias are reported in 38–47% and SCD in 13–18% of adult patients with LVNC.<a class="elsevierStyleCrossRef" href="#bib0010">2</a>Although 80–90% of LVNC patients show ECG abnormalities, no ECG features are specific to the disease.<a class="elsevierStyleCrossRef" href="#bib0015">3</a> Conversely, intraventricular conduction defects are uncommon in children with LVNC, in whom the most frequent arrhythmias or conduction defects are Wolff-Parkinson-White syndrome, AV block (mainly second-degree), VT and bradycardia, while adults usually present with LV hypertrophy, LBBB, VT, atrial fibrillation, QT prolongation and AV block.<a class="elsevierStyleCrossRef" href="#bib0020">4</a> Our patient had LBBB type intra/interventricular conduction defect and first-degree AV block. She also had intermittent RBB block, rare in these patients. Alternating bundle branch block is a class I recommendation, level of evidence C, for cardiac pacing according to the European Society of Cardiology and European Heart Rhythm Association guidelines for cardiac pacing and cardiac resynchronization.The first comprehensive analysis of electrophysiological (EP) findings in a relatively large cohort of patients with LVNC (n=24) proposed that life-threatening ventricular tachyarrhythmias were likely due to the noncompacted myocardium serving as the arrhythmic substrate. Impaired flow reserve in structurally noncompacted myocardial segments with resultant intermittent ischemia could play an important role. However, in that cohort, sustained monomorphic VT was rarely induced, even with isoproterenol infusion. Non-sustained polymorphic VT was observed more commonly and, while it was believed to be nonspecific, three patients with non-sustained polymorphic VT demonstrated malignant ventricular arrhythmias on follow-up. The authors concluded that no specific clinical, electrocardiographic or echocardiographic finding was predictive of VT inducibility, except for the potential protective effect of younger age and LV ejection fraction above 50%. Nevertheless, their findings suggested a negative EPS could identify a subset of patients at low risk of developing malignant tachyarrhythmias.<a class="elsevierStyleCrossRef" href="#bib0025">5</a>The differential diagnosis of a WCT with a typical LBBB morphology is limited to five entities: supraventricular tachycardia (SVT) with fixed LBBB, SVT with functional aberrancy, pre-excited reentrant tachycardias using an atriofascicular accessory pathway as the anterograde limb, SVT with a “bystander” atriofascicular pathway, and BBR-VT. In our patient, the lack of a 1:1 AV relationship excluded a pre-excited reentrant tachycardia using an atriofascicular accessory pathway, while ventricular rate greater than atrial rate strongly suggested a VT (rarely, an AV nodal reentrant tachycardia may present with 2:1 retrograde block, but a longer postpacing interval is typically obtained from the right ventricular apex). Other potential diagnoses were automatic fascicular VT and intramyocardial VT. The former is catecholamine-dependent, not induced with programmed stimulation and shows a variable HV interval in tachycardia, while the latter rarely produces entirely typical RBBB or LBBB patterns on surface ECG and does not depend on a critical delay in the HPS. Therefore, these two diagnoses were considered extremely unlikely.The diagnosis of BBR-VT was suggested by a number of factors:<ul class="elsevierStyleList" id="lis0015"><li class="elsevierStyleListItem" id="lsti0005">•The presence of clear intraventricular conduction abnormalities and first-degree AV block on the baseline ECG;</li><li class="elsevierStyleListItem" id="lsti0010">•A prolonged baseline HV interval;</li><li class="elsevierStyleListItem" id="lsti0015">•The occurrence of a VT with typical LBBB pattern;</li><li class="elsevierStyleListItem" id="lsti0020">•The induction and interruption of the arrhythmia with pacing (suggestive of a reentrant mechanism);</li><li class="elsevierStyleListItem" id="lsti0025">•The need for a critical conduction delay in the HPS for induction of the tachycardia;</li><li class="elsevierStyleListItem" id="lsti0030">•The presence of a clear right bundle deflection preceding each ventricular complex, suggesting the RBB was part of the circuit;</li><li class="elsevierStyleListItem" id="lsti0035">•A short postpacing interval from the right ventricular apex.</li></ul>The current literature contains limited data regarding mapping and ablation of premature ventricular complexes or VT in the presence of LVNC. Fiala et al. described successful ablation of a VT originating in the interventricular septum.<a class="elsevierStyleCrossRef" href="#bib0030">6</a> Derval et al. reported two symptomatic patients who underwent successful radiofrequency ablation of a monomorphic VT in the basolateral aspect of the LV.<a class="elsevierStyleCrossRef" href="#bib0035">7</a> Lim et al. described epicardial ablation of a monomorphic VT located in the epicardial surface of the anterolateral wall.<a class="elsevierStyleCrossRef" href="#bib0040">8</a> The mechanism underlying these arrhythmias is not completely understood. Paparella et al. reconstructed a ventricular electroanatomical mapping in a patient with LVNC and monomorphic VT. No areas of low voltage were identified, probably excluding the presence of scar-based tissue reentry as a possible mechanism.<a class="elsevierStyleCrossRef" href="#bib0045">9</a> There is considerable evidence that most VT episodes in LVNC arise from areas of noncompacted myocardium, and there have been reports that, in some cases, the focus of arrhythmia is not necessarily related to LVNC, indicating the possibility of concomitant idiopathic arrhythmias in this population.<a class="elsevierStyleCrossRef" href="#bib0050">10</a>To the best of our knowledge, no case report of BBR-VT as the first manifestation of LVNC has been published. BBR-VT, an uncommon form of VT incorporating both bundle branches into the reentrant circuit, usually occurs in patients with structural heart disease, mainly dilated cardiomyopathy, although patients with structurally normal hearts have been described. The critical prerequisite for its development is conduction delay in the HPS, which manifests as nonspecific conduction delay or LBBB on the surface ECG and prolonged HV interval in the intracardiac recordings, although some patients may have relatively narrow QRS complexes, suggesting a role of functional conduction delay in the genesis of BBR,<a class="elsevierStyleCrossRef" href="#bib0055">11</a> or even an HV interval within normal limits.<a class="elsevierStyleCrossRef" href="#bib0060">12</a> Patients typically present with presyncope, syncope or SCD because of very rapid rates. QRS morphology during VT is a typical bundle branch block pattern and may be identical to that in sinus rhythm.<a class="elsevierStyleCrossRef" href="#bib0055">11</a>Unlike typical presentations, our patient was practically asymptomatic, reporting mild palpitations despite the incessant nature of her VT (continuing for hours). This lack of symptoms was due to the low rate of the arrhythmia, which is also very unusual for BBR-VT. Preexisting RBB disease or structural cardiac disease (noncompacted myocardium) with more dispersed distal right bundle branches may predispose to such a phenomenon. It may also help explain the occurrence of more than one distinct morphology in BBR-VT utilizing the RBB as the anterograde limb.<a class="elsevierStyleCrossRef" href="#bib0065">13</a> Anterograde conduction occurring in the relative refractory period of a diseased RBB tends to prolong HV interval during VT and could have contributed to its slow and incessant nature.Regarding the VT documented during stress testing, it could have been a left anterior fascicular VT, an uncommon form of fascicular VT which is often induced by exercise. It could have originated in a small reentry region or through triggered automaticity located in the lateral wall (zone of noncompacted myocardium), close to the anterior fascicle of the LBB. As the patient had incomplete RBB block with right axis deviation before and after the occurrence of the arrhythmia, it might have been a BBR-VT with the left anterior fascicle as the anterograde limb and the RBB as the retrograde one, which would explain the fact that this VT had a similar rate as the one induced in the EP lab at the time of left posterior fascicular block.Radiofrequency catheter ablation of a bundle branch can cure BBR-VT and is currently regarded as first-line therapy. The technique of choice is ablation of the RBB. The reported incidence of clinically significant conduction system impairment requiring implantation of a permanent pacemaker is 10–30%.<a class="elsevierStyleCrossRef" href="#bib0055">11</a> The decision not to ablate our patient's right bundle was due to: (1) a high probability of need for chronic ventricular pacing (considering the baseline trifascicular conduction disease and HV prolongation); (2) the asymptomatic nature of her arrhythmia; (3) the possibility of periodic reassessment of the potential benefit of RBB ablation for lowering the number of future ICD shocks or preventing tachycardia-induced cardiomyopathy; ICD monitoring may quantify the number and duration of VT episodes, which will help in this analysis; and (4) ablation of the RBB could result in the easier induction of a less well tolerated interfascicular reentrant VT, as described in the literature.<a class="elsevierStyleCrossRef" href="#bib0070">14</a>Based on current evidence, patients with severely reduced LV ejection fraction should have an ICD implanted empirically and not undergo EPS for risk stratification. Prophylactic ICD implantation appears reasonable in patients with LVNC who fulfill the SCD-HeFT criteria. Patients with LVNC presenting with symptomatic arrhythmias or syncope are also good candidates for ICD implantation.<a class="elsevierStyleCrossRef" href="#bib0075">15</a>Conclusions<ul class="elsevierStyleList" id="lis0020"><li class="elsevierStyleListItem" id="lsti0040">•In patients with LVNC, VT exit points are usually located in regions of noncompacted myocardium. However, BBR-VT may be the first manifestation of this condition.</li><li class="elsevierStyleListItem" id="lsti0045">•BBR-VT is a highly malignant form of VT, often presenting with syncope or SCD. However, in patients with baseline bilateral conduction delay, it may be slow, incessant and exceptionally well tolerated.</li><li class="elsevierStyleListItem" id="lsti0050">•Although catheter ablation of BBR-VT involving both bundle branches has a high success rate in preventing new episodes of the arrhythmia, the decision to perform this procedure must be weighed against potential harm. Ablating the RBB in a young patient with LBB block and HV prolongation will likely result in the need for chronic long-term ventricular pacing.</li><li class="elsevierStyleListItem" id="lsti0055">•The potential usefulness of programming a low tachycardia detection rate and ATP in patients with a history of slow VT for prevention of tachycardia-induced cardiomyopathy must be weighed against the possibility of inappropriate detection and treatment of supraventricular tachycardia (very common in patients with LVNC) and the potential acceleration of a possibly asymptomatic VT resulting in a malignant form of VT. In the present case, ATP failed to terminate the slow VT, resulting in several painful shocks. These events suggest treatment of a slow VT should consist of ATP therapy only or no therapy at all, whereas ATP followed by shocks should be programmed only in the conventional VT zone.</li></ul>Ethical disclosuresProtection of human and animal subjectsThe authors declare that no experiments were performed on humans or animals for this study.Confidentiality of dataThe authors declare that no patient data appear in this article.Right to privacy and informed consentThe authors declare that no patient data appear in this article.Conflicts of interestThe authors have no conflicts of interest to declare." 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The baseline electrocardiogram revealed first-degree atrioventricular block and intraventricular conduction defect. Transthoracic echocardiography showed prominent trabeculae and intertrabecular recesses suggesting left ventricular noncompaction (LVNC), which was confirmed by cardiac magnetic resonance imaging. During electrophysiological study, a sustained bundle branch reentrant VT with LBBB pattern and cycle length of 480 ms, similar to the clinical tachycardia, was easily and reproducibly inducible. As there was considerable risk of need for chronic ventricular pacing following right bundle ablation, no ablation was attempted and a cardioverter-defibrillator was implanted. To the best of our knowledge, no case reports of BBR-VT as the first manifestation of LVNC have been published. Furthermore, this is an extremely rare presentation of BBR-VT, which is usually a highly malignant arrhythmia." ] "pt" => array:2 [ "titulo" => "Resumo" "resumen" => "Uma jovem de quinze anos de idade foi observada em consulta externa de Cardiologia por palpitações ligeiras e documentação de taquicardia ventricular (TV) lenta e incessante com padrão de bloqueio de ramo esquerdo (BRE). O electrocardiograma (ECG) basal revelou bloqueio auriculoventricular (BAV) de primeiro grau e perturbação da condução intraventricular. Um ecocardiograma transtorácico documentou trabeculação proeminente e recessos intertrabeculares, alterações sugestivas de ventrículo esquerdo não-compactado (VENC), diagnóstico confirmado por ressonância magnética cardíaca. No estudo electrofisiológico, uma taquicardia ventricular sustentada por reentrada de ramo, com padrão de BRE e ciclo de base de 480 ms, semelhante à taquicardia clínica, foi repetidamente induzida. Considerando o risco elevado de necessidade de pacing ventricular crónico em caso de ablação do ramo direito (BAV de primeiro grau e BRE no ECG basal e intervalo HV 100 ms no estudo electrofisiológico), não foi efetuado qualquer procedimento ablativo e um cardioversor-desfibrilhador foi implantado. Até ao momento atual, nenhum caso de TV por reentrada de ramo como primeira manifestação de VENC foi publicado. O caso descrito revela uma apresentação extremamente atípica deste tipo de TV, que habitualmente é rápida e maligna." ] ] "multimedia" => array:5 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1112 "Ancho" => 750 "Tamanyo" => 56362 ] ] "descripcion" => array:1 [ "en" => "Cardiac magnetic resonance images suggesting left ventricular noncompaction." ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 1316 "Ancho" => 2468 "Tamanyo" => 597600 ] ] "descripcion" => array:1 [ "en" => "Clinical ventricular tachycardia (LBB block pattern, superior axis)." ] ] 2 => array:7 [ "identificador" => "fig0015" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 1481 "Ancho" => 2500 "Tamanyo" => 476579 ] ] "descripcion" => array:1 [ "en" => "Baseline ECG: sinus rhythm, intraventricular conduction defects and significant PR prolongation." ] ] 3 => array:7 [ "identificador" => "fig0020" "etiqueta" => "Figure 4" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr4.jpeg" "Alto" => 1758 "Ancho" => 2494 "Tamanyo" => 994931 ] ] "descripcion" => array:1 [ "en" => "Inducible sustained VT with LBB block pattern and superior axis and a clear right bundle branch deflection (arrows) preceding each ventricular complex, suggesting the RBB is part of the circuit." ] ] 4 => array:7 [ "identificador" => "fig0025" "etiqueta" => "Figure 5" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr5.jpeg" "Alto" => 1754 "Ancho" => 2460 "Tamanyo" => 641811 ] ] "descripcion" => array:1 [ "en" => "Intracardiac electrogram revealing significant HV interval prolongation (100 ms)." ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:15 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "The morphological spectrum of ventricular noncompaction" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:3 [ 0 => "R.M. 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Case report

Incessant slow bundle branch reentrant ventricular tachycardia in a young patient with left ventricular noncompaction

Taquicardia ventricular por reentrada de ramo lenta e incessante em adolescente com ventrículo esquerdo não-compactado

Sérgio Barraa,

Corresponding author

sergioncbarra@gmail.com

Corresponding author.

, Nuno Morenob, Rui Providênciaa, Helena Gonçalvesc, João José Primoc

a Cardiology Department, Coimbra Hospital and University Centre, Coimbra, Portugal

b Cardiology Department, Padre Américo Hospital Centre, Penafiel, Portugal

c Cardiology Department, V. N. Gaia Hospital Centre, V. N. Gaia, Portugal

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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Bundle branch reentrant ventricular tachycardia &#40;BBR-VT&#41;&#44; an uncommon form of macroreentrant tachycardia&#44; generally occurs in the context of dilated cardiomyopathy&#44; previous valve surgery or other cardiac conditions with underlying His-Purkinje system &#40;HPS&#41; disease&#46; This case is a very unusual presentation of BBR-VT in a young patient with isolated left ventricular noncompaction &#40;LVNC&#41;&#46; Although our patient presented with HPS disease allowing initiation of this arrhythmia&#44; it is rare for BBR-VT to be the first manifestation of isolated LVNC&#46; Furthermore&#44; BBR-VT is a highly malignant arrhythmia&#44; yet our patient was almost asymptomatic due to the surprisingly long cycle length of the ventricular tachycardia &#40;VT&#41; and despite its unusual incessancy&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Case report</span><p id="par0010" class="elsevierStylePara elsevierViewall">A 15-year-old girl was referred to our arrhythmology department for mild palpitations and documented incessant VT &#40;hemodynamically stable VT lasting hours&#41;&#46; She was otherwise healthy&#44; with no relevant medical history and no family history of significant cardiomyopathy or sudden cardiac death &#40;SCD&#41;&#46; Her palpitations were not related to effort and were persistent but otherwise extremely well tolerated&#46; She denied precordial pain&#44; dizziness&#44; or presyncope&#47;syncope&#46; A previous electrocardiogram &#40;ECG&#41; had revealed wide-QRS tachycardia &#40;WCT&#41; at 115 beats per minute &#40;bpm&#41;&#46;</p><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Investigations</span><p id="par0015" class="elsevierStylePara elsevierViewall">The patient was examined shortly after being referred&#44; and denied any symptoms&#44; including palpitations&#46; The physical exam was unremarkable&#46; The first ECG showed a VT with left bundle branch block &#40;LBBB&#41; pattern and superior axis at 118 bpm&#46; A transthoracic echocardiogram revealed a normal-sized LV and preserved overall systolic function&#44; hypertrabeculation of the LV posterior and lateral walls and intertrabecular recesses communicating with the LV cavity as demonstrated by color Doppler flow&#44; suggestive of LVNC&#44; which was confirmed by cardiac magnetic resonance imaging &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>&#41;&#46; Subsequent ECGs alternated between sinus rhythm with intraventricular conduction abnormalities and first-degree atrioventricular &#40;AV&#41; block and slow VT with LBBB pattern and superior axis &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Figure 2</a>&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">An exercise stress test was stopped at 3&#58;58 because of the sudden induction of a well tolerated yet sustained slow wide QRS tachycardia with right bundle branch &#40;RBB&#41; and left posterior fascicular block patterns&#46; During most of the recovery time&#44; an incomplete RBB block pattern with right axis deviation was seen&#44; as at the beginning of the test&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">An electrophysiological study &#40;EPS&#41; was performed&#46; The baseline ECG revealed sinus rhythm&#44; intraventricular conduction defects &#40;QRS 122 ms&#41; and significant PR prolongation &#40;296 ms&#41; &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Figure 3</a>&#41;&#46; A standard protocol using 6-F diagnostic electrophysiology catheters was followed&#46; Two quadripolar catheters were placed in the high right atrium&#44; His bundle and right ventricle as required&#44; and a decapolar catheter in the coronary sinus&#46; The programmed ventricular stimulation protocol included three drive-cycle lengths &#40;CL&#41; and two ventricular extrastimuli while pacing from the right ventricular apex&#46; A 125 bpm-rate monomorphic sustained VT was reproducibly inducible with a single extrastimulus 360 ms after an eight-beat drive-cycle length of 600 ms&#46; It had a LBBB pattern&#44; superior axis and a clear right bundle deflection preceding each ventricular complex&#44; suggesting the RBB was part of the circuit &#40;<a class="elsevierStyleCrossRef" href="#fig0020">Figure 4</a>&#41;&#46; A short postpacing interval was obtained from the right ventricular apex&#46; Left posterior fascicular block was intermittently seen&#44; associated with an increase in the VT CL&#44; further suggesting a diagnosis of BBR-VT&#46; The VT was exceptionally well tolerated and always terminated by anti-tachycardia pacing &#40;ATP&#41; with 380&#8211;420 ms CL&#46; A second VT with a slightly different morphology in the limb leads was also inducible&#46; Short periods of intermittent RBB block were documented during sinus rhythm&#46; Further data included AH interval 157 ms&#59; HV interval 100 ms&#59; and Wenckebach period 540 ms &#40;<a class="elsevierStyleCrossRef" href="#fig0025">Figure 5</a>&#41;&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><elsevierMultimedia ident="fig0020"></elsevierMultimedia><elsevierMultimedia ident="fig0025"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Treatment</span><p id="par0030" class="elsevierStylePara elsevierViewall">As there was a considerable risk of need for chronic long-term ventricular pacing following RBB ablation and considering that this VT was particularly slow and practically asymptomatic&#44; ablation of the RBB was not attempted and the patient was referred for implantation of a dual-chamber cardioverter-defibrillator &#40;ICD&#41;&#46; The rationale for this lay in the spontaneous occurrence of sustained VT&#44; albeit slow&#44; and the easy inducibility of sustained BBR-VT&#44; a highly malignant ventricular tachyarrhythmia that had an unusual presentation in this patient but could nevertheless recur at higher rates&#46; The ICD would protect the patient from potential future episodes of fast VT or ventricular fibrillation and would have additional advantages&#58; &#40;1&#41; ventricular pacing in the event of complete AV block &#40;the patient had significant bilateral bundle branch conduction abnormalities with marked HV prolongation&#41;&#59; and &#40;2&#41; quantification of the number and duration of VT episodes in the upcoming months or years&#46; If persistently high heart rates caused by long episodes of VT are observed&#44; especially if associated with deterioration in LV systolic function &#40;tachycardia-induced cardiomyopathy&#41;&#44; RBB ablation will be considered&#46; In this case&#44; we will then opt for resynchronization&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Outcome and follow-up</span><p id="par0035" class="elsevierStylePara elsevierViewall">During EPS and ICD implantation&#44; ATP successfully terminated all episodes of VT&#46; The defibrillator was accordingly programmed to deliver ATP for VT CL of 400&#8211;500 ms&#46; Termination of episodes of slow VT could help prevent tachycardia-induced cardiomyopathy&#46; A few hours after implantation&#44; the patient received six shocks in a 2-hour period after failed ATP for relatively slow VT &#40;rate 125 bpm&#41;&#44; causing considerable distress to the patient&#44; and ATP was turned off&#46; No further ICD shocks were reported in the following six months and she remains asymptomatic&#46; Several episodes of sustained slow VT have been detected by the ICD&#46;</p></span></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Discussion</span><p id="par0040" class="elsevierStylePara elsevierViewall">LVNC is a rare form of a primary genetic cardiomyopathy considered to be the result of abnormal intrauterine arrest of the myocardial compaction process&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> Heart failure&#44; arrhythmias &#40;including SCD&#41; and embolic events are its classical triad of complications&#46; Ventricular tachyarrhythmias are reported in 38&#8211;47&#37; and SCD in 13&#8211;18&#37; of adult patients with LVNC&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">Although 80&#8211;90&#37; of LVNC patients show ECG abnormalities&#44; no ECG features are specific to the disease&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> Conversely&#44; intraventricular conduction defects are uncommon in children with LVNC&#44; in whom the most frequent arrhythmias or conduction defects are Wolff-Parkinson-White syndrome&#44; AV block &#40;mainly second-degree&#41;&#44; VT and bradycardia&#44; while adults usually present with LV hypertrophy&#44; LBBB&#44; VT&#44; atrial fibrillation&#44; QT prolongation and AV block&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> Our patient had LBBB type intra&#47;interventricular conduction defect and first-degree AV block&#46; She also had intermittent RBB block&#44; rare in these patients&#46; Alternating bundle branch block is a class I recommendation&#44; level of evidence C&#44; for cardiac pacing according to the European Society of Cardiology and European Heart Rhythm Association guidelines for cardiac pacing and cardiac resynchronization&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">The first comprehensive analysis of electrophysiological &#40;EP&#41; findings in a relatively large cohort of patients with LVNC &#40;n&#61;24&#41; proposed that life-threatening ventricular tachyarrhythmias were likely due to the noncompacted myocardium serving as the arrhythmic substrate&#46; Impaired flow reserve in structurally noncompacted myocardial segments with resultant intermittent ischemia could play an important role&#46; However&#44; in that cohort&#44; sustained monomorphic VT was rarely induced&#44; even with isoproterenol infusion&#46; Non-sustained polymorphic VT was observed more commonly and&#44; while it was believed to be nonspecific&#44; three patients with non-sustained polymorphic VT demonstrated malignant ventricular arrhythmias on follow-up&#46; The authors concluded that no specific clinical&#44; electrocardiographic or echocardiographic finding was predictive of VT inducibility&#44; except for the potential protective effect of younger age and LV ejection fraction above 50&#37;&#46; Nevertheless&#44; their findings suggested a negative EPS could identify a subset of patients at low risk of developing malignant tachyarrhythmias&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">The differential diagnosis of a WCT with a typical LBBB morphology is limited to five entities&#58; supraventricular tachycardia &#40;SVT&#41; with fixed LBBB&#44; SVT with functional aberrancy&#44; pre-excited reentrant tachycardias using an atriofascicular accessory pathway as the anterograde limb&#44; SVT with a &#8220;bystander&#8221; atriofascicular pathway&#44; and BBR-VT&#46; In our patient&#44; the lack of a 1&#58;1 AV relationship excluded a pre-excited reentrant tachycardia using an atriofascicular accessory pathway&#44; while ventricular rate greater than atrial rate strongly suggested a VT &#40;rarely&#44; an AV nodal reentrant tachycardia may present with 2&#58;1 retrograde block&#44; but a longer postpacing interval is typically obtained from the right ventricular apex&#41;&#46; Other potential diagnoses were automatic fascicular VT and intramyocardial VT&#46; The former is catecholamine-dependent&#44; not induced with programmed stimulation and shows a variable HV interval in tachycardia&#44; while the latter rarely produces entirely typical RBBB or LBBB patterns on surface ECG and does not depend on a critical delay in the HPS&#46; Therefore&#44; these two diagnoses were considered extremely unlikely&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall">The diagnosis of BBR-VT was suggested by a number of factors&#58;<ul class="elsevierStyleList" id="lis0015"><li class="elsevierStyleListItem" id="lsti0005"><span class="elsevierStyleLabel">&#8226;</span><p id="par0065" class="elsevierStylePara elsevierViewall">The presence of clear intraventricular conduction abnormalities and first-degree AV block on the baseline ECG&#59;</p></li><li class="elsevierStyleListItem" id="lsti0010"><span class="elsevierStyleLabel">&#8226;</span><p id="par0070" class="elsevierStylePara elsevierViewall">A prolonged baseline HV interval&#59;</p></li><li class="elsevierStyleListItem" id="lsti0015"><span class="elsevierStyleLabel">&#8226;</span><p id="par0075" class="elsevierStylePara elsevierViewall">The occurrence of a VT with typical LBBB pattern&#59;</p></li><li class="elsevierStyleListItem" id="lsti0020"><span class="elsevierStyleLabel">&#8226;</span><p id="par0080" class="elsevierStylePara elsevierViewall">The induction and interruption of the arrhythmia with pacing &#40;suggestive of a reentrant mechanism&#41;&#59;</p></li><li class="elsevierStyleListItem" id="lsti0025"><span class="elsevierStyleLabel">&#8226;</span><p id="par0085" class="elsevierStylePara elsevierViewall">The need for a critical conduction delay in the HPS for induction of the tachycardia&#59;</p></li><li class="elsevierStyleListItem" id="lsti0030"><span class="elsevierStyleLabel">&#8226;</span><p id="par0090" class="elsevierStylePara elsevierViewall">The presence of a clear right bundle deflection preceding each ventricular complex&#44; suggesting the RBB was part of the circuit&#59;</p></li><li class="elsevierStyleListItem" id="lsti0035"><span class="elsevierStyleLabel">&#8226;</span><p id="par0095" class="elsevierStylePara elsevierViewall">A short postpacing interval from the right ventricular apex&#46;</p></li></ul></p><p id="par0100" class="elsevierStylePara elsevierViewall">The current literature contains limited data regarding mapping and ablation of premature ventricular complexes or VT in the presence of LVNC&#46; Fiala et al&#46; described successful ablation of a VT originating in the interventricular septum&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> Derval et al&#46; reported two symptomatic patients who underwent successful radiofrequency ablation of a monomorphic VT in the basolateral aspect of the LV&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> Lim et al&#46; described epicardial ablation of a monomorphic VT located in the epicardial surface of the anterolateral wall&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a> The mechanism underlying these arrhythmias is not completely understood&#46; Paparella et al&#46; reconstructed a ventricular electroanatomical mapping in a patient with LVNC and monomorphic VT&#46; No areas of low voltage were identified&#44; probably excluding the presence of scar-based tissue reentry as a possible mechanism&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a> There is considerable evidence that most VT episodes in LVNC arise from areas of noncompacted myocardium&#44; and there have been reports that&#44; in some cases&#44; the focus of arrhythmia is not necessarily related to LVNC&#44; indicating the possibility of concomitant idiopathic arrhythmias in this population&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">10</span></a></p><p id="par0105" class="elsevierStylePara elsevierViewall">To the best of our knowledge&#44; no case report of BBR-VT as the first manifestation of LVNC has been published&#46; BBR-VT&#44; an uncommon form of VT incorporating both bundle branches into the reentrant circuit&#44; usually occurs in patients with structural heart disease&#44; mainly dilated cardiomyopathy&#44; although patients with structurally normal hearts have been described&#46; The critical prerequisite for its development is conduction delay in the HPS&#44; which manifests as nonspecific conduction delay or LBBB on the surface ECG and prolonged HV interval in the intracardiac recordings&#44; although some patients may have relatively narrow QRS complexes&#44; suggesting a role of functional conduction delay in the genesis of BBR&#44;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">11</span></a> or even an HV interval within normal limits&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">12</span></a> Patients typically present with presyncope&#44; syncope or SCD because of very rapid rates&#46; QRS morphology during VT is a typical bundle branch block pattern and may be identical to that in sinus rhythm&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">11</span></a></p><p id="par0110" class="elsevierStylePara elsevierViewall">Unlike typical presentations&#44; our patient was practically asymptomatic&#44; reporting mild palpitations despite the incessant nature of her VT &#40;continuing for hours&#41;&#46; This lack of symptoms was due to the low rate of the arrhythmia&#44; which is also very unusual for BBR-VT&#46; Preexisting RBB disease or structural cardiac disease &#40;noncompacted myocardium&#41; with more dispersed distal right bundle branches may predispose to such a phenomenon&#46; It may also help explain the occurrence of more than one distinct morphology in BBR-VT utilizing the RBB as the anterograde limb&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">13</span></a> Anterograde conduction occurring in the relative refractory period of a diseased RBB tends to prolong HV interval during VT and could have contributed to its slow and incessant nature&#46;</p><p id="par0115" class="elsevierStylePara elsevierViewall">Regarding the VT documented during stress testing&#44; it could have been a left anterior fascicular VT&#44; an uncommon form of fascicular VT which is often induced by exercise&#46; It could have originated in a small reentry region or through triggered automaticity located in the lateral wall &#40;zone of noncompacted myocardium&#41;&#44; close to the anterior fascicle of the LBB&#46; As the patient had incomplete RBB block with right axis deviation before and after the occurrence of the arrhythmia&#44; it might have been a BBR-VT with the left anterior fascicle as the anterograde limb and the RBB as the retrograde one&#44; which would explain the fact that this VT had a similar rate as the one induced in the EP lab at the time of left posterior fascicular block&#46;</p><p id="par0120" class="elsevierStylePara elsevierViewall">Radiofrequency catheter ablation of a bundle branch can cure BBR-VT and is currently regarded as first-line therapy&#46; The technique of choice is ablation of the RBB&#46; The reported incidence of clinically significant conduction system impairment requiring implantation of a permanent pacemaker is 10&#8211;30&#37;&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">11</span></a> The decision not to ablate our patient&#39;s right bundle was due to&#58; &#40;1&#41; a high probability of need for chronic ventricular pacing &#40;considering the baseline trifascicular conduction disease and HV prolongation&#41;&#59; &#40;2&#41; the asymptomatic nature of her arrhythmia&#59; &#40;3&#41; the possibility of periodic reassessment of the potential benefit of RBB ablation for lowering the number of future ICD shocks or preventing tachycardia-induced cardiomyopathy&#59; ICD monitoring may quantify the number and duration of VT episodes&#44; which will help in this analysis&#59; and &#40;4&#41; ablation of the RBB could result in the easier induction of a less well tolerated interfascicular reentrant VT&#44; as described in the literature&#46;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">14</span></a></p><p id="par0125" class="elsevierStylePara elsevierViewall">Based on current evidence&#44; patients with severely reduced LV ejection fraction should have an ICD implanted empirically and not undergo EPS for risk stratification&#46; Prophylactic ICD implantation appears reasonable in patients with LVNC who fulfill the SCD-HeFT criteria&#46; Patients with LVNC presenting with symptomatic arrhythmias or syncope are also good candidates for ICD implantation&#46;<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">15</span></a></p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Conclusions</span><p id="par0130" class="elsevierStylePara elsevierViewall"><ul class="elsevierStyleList" id="lis0020"><li class="elsevierStyleListItem" id="lsti0040"><span class="elsevierStyleLabel">&#8226;</span><p id="par0135" class="elsevierStylePara elsevierViewall">In patients with LVNC&#44; VT exit points are usually located in regions of noncompacted myocardium&#46; However&#44; BBR-VT may be the first manifestation of this condition&#46;</p></li><li class="elsevierStyleListItem" id="lsti0045"><span class="elsevierStyleLabel">&#8226;</span><p id="par0140" class="elsevierStylePara elsevierViewall">BBR-VT is a highly malignant form of VT&#44; often presenting with syncope or SCD&#46; However&#44; in patients with baseline bilateral conduction delay&#44; it may be slow&#44; incessant and exceptionally well tolerated&#46;</p></li><li class="elsevierStyleListItem" id="lsti0050"><span class="elsevierStyleLabel">&#8226;</span><p id="par0145" class="elsevierStylePara elsevierViewall">Although catheter ablation of BBR-VT involving both bundle branches has a high success rate in preventing new episodes of the arrhythmia&#44; the decision to perform this procedure must be weighed against potential harm&#46; Ablating the RBB in a young patient with LBB block and HV prolongation will likely result in the need for chronic long-term ventricular pacing&#46;</p></li><li class="elsevierStyleListItem" id="lsti0055"><span class="elsevierStyleLabel">&#8226;</span><p id="par0150" class="elsevierStylePara elsevierViewall">The potential usefulness of programming a low tachycardia detection rate and ATP in patients with a history of slow VT for prevention of tachycardia-induced cardiomyopathy must be weighed against the possibility of inappropriate detection and treatment of supraventricular tachycardia &#40;very common in patients with LVNC&#41; and the potential acceleration of a possibly asymptomatic VT resulting in a malignant form of VT&#46; In the present case&#44; ATP failed to terminate the slow VT&#44; resulting in several painful shocks&#46; These events suggest treatment of a slow VT should consist of ATP therapy only or no therapy at all&#44; whereas ATP followed by shocks should be programmed only in the conventional VT zone&#46;</p></li></ul></p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Ethical disclosures</span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">Protection of human and animal subjects</span><p id="par0155" class="elsevierStylePara elsevierViewall">The authors declare that no experiments were performed on humans or animals for this study&#46;</p></span><span id="sec0050" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0070">Confidentiality of data</span><p id="par0160" class="elsevierStylePara elsevierViewall">The authors declare that no patient data appear in this article&#46;</p></span><span id="sec0055" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0075">Right to privacy and informed consent</span><p id="par0165" class="elsevierStylePara elsevierViewall">The authors declare that no patient data appear in this article&#46;</p></span></span><span id="sec0060" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0080">Conflicts of interest</span><p id="par0170" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare&#46;</p></span></span>"
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            0 => "Left ventricular noncompaction"
            1 => "Bundle branch reentrant ventricular tachycardia"
            2 => "Electrophysiologic study"
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            0 => "Ventr&#237;culo esquerdo n&#227;o-compactado"
            1 => "Taquicardia ventricular por reentrada de ramo"
            2 => "Estudo electrofisiol&#243;gico"
            3 => "Cardioversor-desfibrilhador implant&#225;vel"
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        "resumen" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A 15-year-old girl was admitted to the cardiology outpatient clinic due to mild palpitations and documented incessant slow ventricular tachycardia &#40;VT&#41; with left bundle branch block &#40;LBBB&#41; pattern&#46; The baseline electrocardiogram revealed first-degree atrioventricular block and intraventricular conduction defect&#46; Transthoracic echocardiography showed prominent trabeculae and intertrabecular recesses suggesting left ventricular noncompaction &#40;LVNC&#41;&#44; which was confirmed by cardiac magnetic resonance imaging&#46; During electrophysiological study&#44; a sustained bundle branch reentrant VT with LBBB pattern and cycle length of 480 ms&#44; similar to the clinical tachycardia&#44; was easily and reproducibly inducible&#46; As there was considerable risk of need for chronic ventricular pacing following right bundle ablation&#44; no ablation was attempted and a cardioverter-defibrillator was implanted&#46; To the best of our knowledge&#44; no case reports of BBR-VT as the first manifestation of LVNC have been published&#46; Furthermore&#44; this is an extremely rare presentation of BBR-VT&#44; which is usually a highly malignant arrhythmia&#46;</p>"
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        "resumen" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Uma jovem de quinze anos de idade foi observada em consulta externa de Cardiologia por palpita&#231;&#245;es ligeiras e documenta&#231;&#227;o de taquicardia ventricular &#40;TV&#41; lenta e incessante com padr&#227;o de bloqueio de ramo esquerdo &#40;BRE&#41;&#46; O electrocardiograma &#40;ECG&#41; basal revelou bloqueio auriculoventricular &#40;BAV&#41; de primeiro grau e perturba&#231;&#227;o da condu&#231;&#227;o intraventricular&#46; Um ecocardiograma transtor&#225;cico documentou trabecula&#231;&#227;o proeminente e recessos intertrabeculares&#44; altera&#231;&#245;es sugestivas de ventr&#237;culo esquerdo n&#227;o-compactado &#40;VENC&#41;&#44; diagn&#243;stico confirmado por resson&#226;ncia magn&#233;tica card&#237;aca&#46; No estudo electrofisiol&#243;gico&#44; uma taquicardia ventricular sustentada por reentrada de ramo&#44; com padr&#227;o de BRE e ciclo de base de 480 ms&#44; semelhante &#224; taquicardia cl&#237;nica&#44; foi repetidamente induzida&#46; Considerando o risco elevado de necessidade de <span class="elsevierStyleItalic">pacing</span> ventricular cr&#243;nico em caso de abla&#231;&#227;o do ramo direito &#40;BAV de primeiro grau e BRE no ECG basal e intervalo HV 100 ms no estudo electrofisiol&#243;gico&#41;&#44; n&#227;o foi efetuado qualquer procedimento ablativo e um cardioversor-desfibrilhador foi implantado&#46; At&#233; ao momento atual&#44; nenhum caso de TV por reentrada de ramo como primeira manifesta&#231;&#227;o de VENC foi publicado&#46; O caso descrito revela uma apresenta&#231;&#227;o extremamente at&#237;pica deste tipo de TV&#44; que habitualmente &#233; r&#225;pida e maligna&#46;</p>"
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Article information

ISSN: 21742049
Original language: English

DOI:10.1016/j.repce.2013.07.004

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Year/Month	Html	Pdf	Total

2024 November	10	12	22
2024 October	48	36	84
2024 September	76	27	103
2024 August	74	34	108
2024 July	46	31	77
2024 June	34	24	58
2024 May	69	22	91
2024 April	60	44	104
2024 March	65	22	87
2024 February	58	23	81
2024 January	50	29	79
2023 December	57	36	93
2023 November	64	32	96
2023 October	65	18	83
2023 September	41	21	62
2023 August	74	23	97
2023 July	62	14	76
2023 June	53	14	67
2023 May	88	28	116
2023 April	71	7	78
2023 March	84	26	110
2023 February	68	29	97
2023 January	56	14	70
2022 December	71	27	98
2022 November	73	32	105
2022 October	59	14	73
2022 September	61	45	106
2022 August	59	45	104
2022 July	59	34	93
2022 June	55	28	83
2022 May	67	38	105
2022 April	66	32	98
2022 March	50	37	87
2022 February	88	35	123
2022 January	155	35	190
2021 December	59	38	97
2021 November	62	39	101
2021 October	90	43	133
2021 September	69	30	99
2021 August	78	36	114
2021 July	56	34	90
2021 June	62	25	87
2021 May	63	50	113
2021 April	185	64	249
2021 March	147	21	168
2021 February	109	13	122
2021 January	95	14	109
2020 December	93	13	106
2020 November	109	21	130
2020 October	64	19	83
2020 September	82	14	96
2020 August	77	11	88
2020 July	61	7	68
2020 June	80	6	86
2020 May	101	16	117
2020 April	71	11	82
2020 March	99	10	109
2020 February	175	20	195
2020 January	55	9	64
2019 December	75	4	79
2019 November	57	6	63
2019 October	42	7	49
2019 September	100	10	110
2019 August	42	6	48
2019 July	42	16	58
2019 June	42	7	49
2019 May	50	30	80
2019 April	43	19	62
2019 March	124	21	145
2019 February	97	16	113
2019 January	92	7	99
2018 December	110	14	124
2018 November	151	16	167
2018 October	437	33	470
2018 September	151	8	159
2018 August	123	7	130
2018 July	59	5	64
2018 June	60	7	67
2018 May	95	12	107
2018 April	101	7	108
2018 March	90	7	97
2018 February	57	5	62
2018 January	121	5	126
2017 December	89	9	98
2017 November	62	19	81
2017 October	58	13	71
2017 September	42	14	56
2017 August	41	17	58
2017 July	36	12	48
2017 June	80	17	97
2017 May	104	21	125
2017 April	53	3	56
2017 March	83	19	102
2017 February	143	7	150
2017 January	67	3	70
2016 December	57	7	64
2016 November	67	4	71
2016 October	100	8	108
2016 September	142	8	150
2016 August	64	6	70
2016 July	39	3	42
2016 June	44	1	45
2016 May	53	6	59
2016 April	26	2	28
2016 March	47	12	59
2016 February	45	20	65
2016 January	30	14	44
2015 December	35	16	51
2015 November	33	12	45
2015 October	45	9	54
2015 September	45	9	54
2015 August	51	5	56
2015 July	46	6	52
2015 June	29	4	33
2015 May	40	4	44
2015 April	34	14	48
2015 March	26	8	34
2015 February	33	6	39
2015 January	36	3	39
2014 December	39	16	55
2014 November	28	3	31
2014 October	37	9	46
2014 September	32	8	40
2014 August	32	6	38
2014 July	38	11	49
2014 June	28	6	34
2014 May	28	18	46
2014 April	22	4	26
2014 March	45	17	62
2014 February	46	8	54
2014 January	63	12	75
2013 December	46	8	54
2013 November	61	12	73
2013 October	73	9	82
2013 September	52	18	70
2013 August	57	17	74

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